Interstitial lung disease (ILD) is considered the most frequent and serious pulmonary complication in primary Sjögren's syndrome (pSS), with the majority of the studies indicating a prevalence of about 20%, and resulting in significant morbidity and mortality.
The pulmonary manifestations of Sjögren's syndrome include airway abnormalities, interstitial lung disease (ILD) and lymphoproliferative disorders (table 1). Lung involvement occurs in ∼9–20% of patients. Subclinical lung disease is even more frequent, including small airway disease and airway inflammation [5].
ACTION STEPS for Sjogren's patients
Always tell your rheumatologist if you are experiencing possible lung disease symptoms, even if they are mild. Examples include: chronic cough, wheezing, chest burning/ tightness, and shortness of breath, especially on exertion.
Background: Primary Sjögren syndrome (pSS) is a chronic inflammatory autoimmune disease that is characterized by lymphocytic infiltration of the exocrine glands and extraglandular organ systems. Interstitial lung disease (ILD) is common in pSS patients and is one of the independent risk factors for a poor prognosis.
Interstitial lung disease (ILD) may cause life-threatening complications of primary Sjogren's syndrome (pSS), and has a poor prognosis in terms of survival and quality of life.
There's no cure for interstitial lung disease. Treatment for ILD usually focuses on treating underlying disease and improving your symptoms. Your healthcare provider might prescribe physical therapy, supplemental oxygen or medication to reduce inflammation or slow down the disease.
ILDs can be mild, serious, or even life-threatening. Symptoms of ILDs may include shortness of breath, dry cough, chest discomfort, and extreme tiredness. Your healthcare provider may diagnose your ILD based on your medical and family histories and results from lung tests, blood tests, and genetic testing.
Interstitial lung disease can be caused by long-term exposure to hazardous materials, such as asbestos. Some types of autoimmune diseases, such as rheumatoid arthritis, also can cause interstitial lung disease. In some cases, however, the causes remain unknown. Once lung scarring occurs, it's generally irreversible.
The disorder can affect the nervous system, muscles, joints, kidneys, lungs, blood vessels, liver, and pancreas. Sjögren's syndrome is a long-lasting disorder that affects females more often than men.
Avoiding foods that tend to exacerbate symptoms including alcohol, caffeine, acidic foods, and spicy foods.
Patients with Sjogren's disease have destruction of the mucus secreting cells in the bronchi. This manifestation of the disease leads to the common complaint of persistent dry cough that is seen in many of these patients.
Hydroxychloroquine (Plaquenil), a drug designed to treat malaria, is often helpful in treating Sjogren's syndrome. Drugs that suppress the immune system, such as methotrexate (Trexall), also might be prescribed.
If Sjögren's syndrome isn't treated appropriately, significant, long-term complications could result that affect your eyes, mouth, lungs, kidneys, liver or lymph nodes — complications including blindness, significant dental destruction and non-Hodgkin lymphoma.
In most people with Sjögren syndrome, dry eyes and dry mouth are the primary features of the disorder, and general health and life expectancy are largely unaffected.
There is no cure for Sjögren's syndrome, but treatments can relieve symptoms. Depending on your specific issues, your doctor may recommend one or more of these therapies.
The most common symptom of all ILDs is shortness of breath. This is often accompanied by a dry cough, chest discomfort, fatigue and occasionally weight loss. In most cases, by the time the symptoms appear lung damage has already been done so it is important to see your doctor immediately.
Computerized tomography (CT) scan.
This imaging test is key to, and sometimes the first step in, the diagnosis of interstitial lung disease. CT scanners use a computer to combine X-ray images taken from many different angles to produce cross-sectional images of internal structures.
Nonspecific interstitial pneumonia has only been defined for a relatively short time, and less is known about how common it is versus other kinds of interstitial pneumonia. Caucasians appear to account for most of the cases, with an average age of onset between 40 and 50 years.
Novel Treatments to Slow Disease
ILD with no known cause is called idiopathic pulmonary fibrosis (IPF). If you have IPF, your doctor may recommend one of two drugs that slow down certain types of scarring: pirfenidone (Esbriet®) and nintedanib (Ofev®).
Family history: You have a higher risk of ILDs if a close relative has an ILD. Mutations, or changes in your genes, can make you more likely to develop scar tissue in your lungs. Environment and occupation: Exposure to mold, chemicals, or bacteria at work or in your environment can damage your lungs.
Exercise is one of the few treatments to show positive changes in functional capacity and symptoms. We have previously shown that exercise training could significantly improve exercise capacity and reduce dyspnoea and fatigue symptoms in patients with ILD of varying aetiology [17].